Ion transporters and channels Flashcards Preview

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Flashcards in Ion transporters and channels Deck (8)
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1
Q

Describe the action of lamotrogene (AED)

A
  • Accesses the voltage-gated sodium channels through fenestration’s (deeply buried binding site)
  • The drug is buried deep inside the protein
  • Inhibit Voltage-gated sodium channels
  • Extend the inactivation period
2
Q

Describe some channelopathies of voltage-gated sodium channels (NAv)

A
  • SCN1A - epilepsy, migraine, autism
  • SCN2A - epilepsy, autism, episodic ataxia
  • SCN3A - epilepsy
  • SCN9A - pain insensitivity and extreme pain disorder
3
Q

Describe some potassium channelopathies

A
  • KCNQ2, KCNQ3, KCNMA1 associated epilepsy syndromes

- KCNA1 associated with episodic ataxia type 1

4
Q

Describe some calcium channelopathies

A
  • CACNA1A episodic ataxia type 2, SCA6 and familial hemiplegic migraine
  • CACNA1B myoclonus-dystonia syndrome
  • CACNA1F X-linked congenital stationary night blindness
  • CACNA1H childhood absence epilepsy
5
Q

Describe how channelopathies work

A
  • mutations can affect channel assembly and or function, pore permeability and inactivation
6
Q

Describe the channelopathy for SCA6 (spinocerebellar ataxia type 6)

A
  • Nucleotide repeat of 16-18 glut amines in a row which inactivates the calcium channel
7
Q

What is dominant-negative channelopathy

A

Where you may have only one single mutation that is enough to poison the whole complex and prevent it from functioning

8
Q

Transient receptor potential channels

A
  • non-selective cation channels

- can be gated by temperature, ligands (nitric oxide) or chemical stress